Growth, Body Composition, and Strength of Children With Cystic Fibrosis Treated With Elexacaftor/Tezacaftor/Ivacaftor (ETI).

Objective: We prospectively monitored rates of change for growth, body mass and composition, muscle strength, and FEV1 in 6-11-year-olds initiating ETI therapy, comparing them to those of US reference children. We assessed factors potentially contributing to rate of change and report ranges of individual variation.

Methods: Body composition was assessed using bioelectrical impedance analysis (BIA), and rates of change were analyzed using linear mixed effects regression models.

Hemostatic derangements associated with cardiopulmonary bypass predict outcomes in pediatric patients undergoing corrective heart surgery.

Background: Understanding of the hemostatic and complement alterations associated with cardiopulmonary bypass (CPB) in pediatric patients and the impact of these alterations on outcome is limited.

Objectives: The present study prospectively characterized these alterations and their association with postoperative outcomes in pediatric CPB.

Methods: All patients aged <21 years undergoing CPB at the authors' institution between 2020 and 2021 who weighed >3 kg, were >36 weeks gestational age, and had no known prothrombotic or hemorrhagic disorders were eligible.

World Allergy Organization (WAO) Diagnosis and Rationale for Action against Cow's Milk Allergy (DRACMA) guideline update - XI - Milk supplement/replacement formulas for infants and toddlers with CMA - Systematic review.

Background: Cow's milk allergy (CMA) is the most complex and common food allergy in infants. Elimination of cow's milk from the diet and replacement with a specialized formula for infants with cow's milk allergy who cannot be breastfed is an established approach to minimize the risk of severe allergic reactions while avoiding nutritional deficiencies. Given the availability of multiple options, such as extensively hydrolyzed cow's milk-based formula (eHF-CM), aminoacid formula (AAF), hydrolyzed rice formula (HRF), and soy formula (SF), there is some uncertainty regarding which formula might represent the most suitable choice with respect to health outcomes.

Differences in disease characteristics and treatment exposures between paediatric and adult-onset inflammatory bowel disease using a registry-based cohort.

Background: Previous studies highlighted a more extensive phenotype for paediatric-onset than adult-onset inflammatory bowel disease (IBD). However, most lacked long-term follow-up, and some were conducted before the era of biologics.

Aims: The aim of this study is to compare disease characteristics and treatment exposures between paediatric-onset and adult-onset IBD.

Eicosatetraynoic Acid Regulates Pro-Fibrotic Pathways in an Induced Pluripotent Stem Cell Derived Macrophage:Human Intestinal Organoid Model of Crohn's Disease.

Background And Aims: We previously identified small molecules predicted to reverse an ileal gene signature for future Crohn's Disease (CD) strictures. Here we used a new human intestinal organoid (HIO) model system containing macrophages to test a lead candidate, eicosatetraynoic acid (ETYA).

Methods: Induced pluripotent stem cell lines (iPSC) were derived from CD patients and differentiated into macrophages and HIOs.

Long-term metabolic effects of non-nutritive sweeteners.

Objective: Excessive consumption of added sugars has been linked to the rise in obesity and associated metabolic abnormalities. Non-nutritive sweeteners (NNSs) offer a potential solution to reduce sugar intake, yet their metabolic safety remains debated. This study aimed to systematically assess the long-term metabolic effects of commonly used NNSs under both normal and obesogenic conditions.

Editorial: New Insights Into the Complexities and Neurocognitive Correlates of Sleep in Adolescents with Attention-Deficit/Hyperactivity Disorder.

Despite decades-long interest in sleep in individuals with attention-deficit/hyperactivity disorder (ADHD)-"restless sleep" was a symptom for diagnosing attention deficit disorder in the Third Edition of the DSM-our understanding of its role in the pathophysiology, trajectories, and functional outcomes of ADHD remains far from complete. This is perhaps no more so the case than in the developmental period of adolescence. The oft-described "perfect storm" of short and ill-timed sleep coinciding with bioregulatory maturation during adolescence may be further intensified for adolescents with ADHD, who often experience wide-ranging and intensifying functional impairments.

Complex Regulation of Protocadherin Epigenetics on Aging-Related Brain Health.

Life expectancy continues to increase in the high-income world due to advances in medical care; however, quality of life declines with increasing age due to normal aging processes. Current research suggests that various aspects of aging are genetically modulated and thus may be slowed via genetic modification. Here, we show evidence for epigenetic modulation of the aging process in the brain from over 1800 individuals as part of the Framingham Heart Study.

Leukopenia, neutropenia, and procalcitonin levels in young febrile infants with invasive bacterial infections.

Background And Objective: Serum procalcitonin (PCT) is a highly accurate biomarker for stratifying the risk of invasive bacterial infections (IBIs) in febrile infants ≤60 days old. However, PCT is unavailable in some settings. We explored the association of leukopenia and neutropenia with IBIs in non-critically ill febrile infants ≤60 days old, with and without PCT.

Unmasking the Impact of Oxygenator-Induced Hypocapnia on Blood Lactate in Veno-Arterial Extracorporeal Membrane Oxygenation.

Extracorporeal membrane oxygenation (ECMO) is often associated with disturbances in acid/base status that can be triggered by the underlying pathology or the ECMO circuit itself. Extracorporeal membrane oxygenation is known to cause hypocapnia, but the impact of reduced partial pressure of carbon dioxide (pCO 2 ) on biomarkers of tissue perfusion during veno-arterial (VA)-ECMO has not been evaluated. To study the impact of low pCO 2 on perfusion indices in VA-ECMO, we placed Sprague-Dawley rats on an established VA-ECMO circuit using either an oxygen/carbon dioxide mixture (O 2 95%, CO 2 5%) or 100% O 2 delivered through the oxygenator (n = 5 per cohort).

Linking microbial genes to plasma and stool metabolites uncovers host-microbial interactions underlying ulcerative colitis disease course.

Understanding the role of the microbiome in inflammatory diseases requires the identification of microbial effector molecules. We established an approach to link disease-associated microbes to microbial metabolites by integrating paired metagenomics, stool and plasma metabolomics, and culturomics. We identified host-microbial interactions correlated with disease activity, inflammation, and the clinical course of ulcerative colitis (UC) in the Predicting Response to Standardized Colitis Therapy (PROTECT) pediatric inception cohort.

S. aureus drives itch and scratch-induced skin damage through a V8 protease-PAR1 axis.

Itch is an unpleasant sensation that evokes a desire to scratch. The skin barrier is constantly exposed to microbes and their products. However, the role of microbes in itch generation is unknown.

Association Between Urinary Biomarkers and CKD in Extremely Low Gestational Age Neonates.

Rationale & Objective: Children born before 28 weeks' gestation are at increased risk of chronic kidney disease (CKD). Urine biomarkers may shed light on mechanistic pathways and improve the ability to forecast CKD. We evaluated whether urinary biomarkers in neonates of low gestational age (GA) are associated with a reduced estimated glomerular filtration rate (eGFR) over time.

Practice Variations in Managing Infantile Hemangiomas.

Infantile hemangioma (IH) is the most common benign tumor of infancy. For children with IH who require treatment, propranolol and other beta blockers have been shown to be safe and effective. Although consensus guidelines for managing IH have been published, anecdotal experience suggests that there remain variations in management.

The Cystic Fibrosis Learning Network: A mixed methods evaluation of program goals, attributes, and impact.

Introduction: The Cystic Fibrosis (CF) Foundation sponsored the design, pilot testing, and implementation of the CF Learning Network (CFLN) to explore how the Foundation's Care Center Network (CCN) could become a learning health system. Six years after the design, the Foundation commissioned a formative mixed methods evaluation of the CFLN to assess: CFLN participants' understanding of program goals, attributes, and perceptions of current and future impact.

Methods: We performed semi-structured interviews with CFLN participants to identify perceived goals, attributes, and impact of the network.

Factor XII promotes the thromboinflammatory response in a rat model of venoarterial extracorporeal membrane oxygenation.

Background: Factor XII (FXII) is a multifunctional protease capable of activating thrombotic and inflammatory pathways. FXII has been linked to thrombosis in extracorporeal membrane oxygenation (ECMO), but the role of FXII in ECMO-induced inflammatory complications has not been studied. We used novel gene-targeted FXII- deficient rats to evaluate the role of FXII in ECMO-induced thromboinflammation.

Accumulation of immune-suppressive CD4 + T cells in aging - tempering inflammaging at the expense of immunity.

The 'immune risk profile' has been shown to predict mortality in the elderly, highlighting the need to better understand age-related immune dysfunction. While aging leads to many defects affecting all arms of the immune system, this review is focused on the accrual of immuno-suppressive CD4 + T cell populations, including FoxP3 + regulatory T cells, and subsets of IL-10-producing T follicular helper cells. New data suggest that such accumulations constitute feedback mechanisms to temper the ongoing progressive low-grade inflammation that develops with age, the so-called "inflammaging", and by doing so, how they have the potential to promote healthier aging.